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EBV induced Evans syndrome in a patient of SLE related MAS: A case report

WeiBin Li1,2,3, Yini Chen1, Ya Chen1

  • 1Department of Clinical Laboratory Medicine, 900th Hospital of PLA Joint Logistic Support Force, Fuzhou, Fujian, China.

Medicine
|August 22, 2026
PubMed
Abstract

Insights

Epstein-Barr virus (EBV) triggered Evans syndrome in systemic lupus erythematosus (SLE) with macrophage activation syndrome (MAS) is rare. Early diagnosis and treatment are crucial for managing this condition.

Area of Science:

  • Rheumatology
  • Pediatric Immunology
  • Infectious Diseases

Background:

  • Epstein-Barr virus (EBV) induced Evans syndrome in systemic lupus erythematosus (SLE) related macrophage activation syndrome (MAS) is a rare condition.
  • This syndrome has a low incidence (0.9%–9%) but a significant mortality rate (4%–19%), often being misdiagnosed as hemophagocytic lymphohistiocytosis.
  • Early recognition is critical due to potential misdiagnosis and high mortality.

Purpose of the Study:

  • To report a rare case of EBV-induced Evans syndrome in a patient with SLE-related MAS.
  • To highlight the diagnostic challenges and successful management of this complex condition.
  • To emphasize the importance of differential diagnosis in similar clinical presentations.

Main Methods:

  • A 15-year-old female presented with fever, abdominal pain, butterfly rash, and lymphadenopathy.
  • Physical examination revealed pharyngeal congestion, tonsil swelling, and hepatomegaly.
  • Diagnosis was confirmed through complete blood count, biochemical, immunologic examinations, and bone marrow cytology, identifying EBV-induced Evans syndrome in SLE-related MAS.

Main Results:

  • The patient received a combination of antibiotics, antivirals, corticosteroids, and immunoglobulins.
  • After 20 days of treatment, the patient showed significant clinical improvement and was discharged.
  • Regular follow-up was established to monitor the patient's condition.

Conclusions:

  • Accurate diagnosis of EBV-induced Evans syndrome in SLE-related MAS relies on a comprehensive evaluation of disease history, laboratory findings, and differential diagnosis.
  • Prompt and appropriate treatment can lead to significant improvement and successful outcomes.
  • This case underscores the need for heightened awareness among clinicians regarding this rare but serious condition.
Keywords:
Evans syndromehemophagocytic syndromemacrophage activation syndromesystemic lupus erythematosus

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